3. Interconversion of glucose-1-phosphate and glucose-6-phosphate is an importan

Question

3. Interconversion of glucose-1-phosphate and glucose-6-phosphate is an important step for both glycogen synthesis and glycogen utilization. Deficiency in the enzyme catalyzing this reaction (PGM1 in most cases) leads to problems in glucose utilization by liver and muscle, especially glycogen synthesis. Impaired glycogen synthesis because of this defect can be ameliorated partially by supplementing diet with a specific monomeric sugar Name this sugar and explain how this supplementation helps with PGM1 deficiency in biochemical terms. (8 points)

Explanation / Answer

1. Phosphoglucomutase 1 is an important enzyme in glycogen synthesis pathway. It converts glucose 6 phosphate into glucose 1 phosphate which will be converted to UDP glucose by UDP glucose pyrophosporylase and then UDP glucose will be added to nascent glucose chains to form glycogen

2. If phosphoglucomutase 1 enzyme is deficient,liver or muscle can't store their glycogen because of lack of glycogen synthesis

3. D galactose can be added as suppliment in diet for persons having PGM 1 deficiency. Since UDP glucose and galactose metabolism are closely related. It can improve protein glycosylation if UDP glucose is not present.

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